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Background: The growing number of adult patients after the Fontan operation requires regular surveillance tests in specialized centers., Aims: Our study aimed to evaluate the current practice of care for Fontan patients in Poland using a multicenter survey., Methods: Eight centers were included in the study including 5 adult congenital heart disease (ACHD) and 3 pediatric centers for adolescents. To compare the centers and facilitate interpretation of results, the Fontan Surveillance Score (FSS) was developed. The higher score is consistent with better care, with a maximum of 19 points., Results: We included in the study 398 Fontan patients (243 adults and 155 adolescents [aged 14-18 years]). The median FSS was 13 points with variability between centers (interquartile range 7-14 points). Centers providing continuous care from the pediatric period until 18 years of age achieved a higher FSS compared to ACHD centers (median: 14 points vs. 12 points; P <0.001). Most of the patients, both in the ACHD (82.3%) and pediatric centers (89%), were seen annually and had a physical examination, electrocardiogram, and echocardiogram performed at each visit. However, we observed unsatisfactory utilization of tests identifying early stages of Fontan circulation failure (cardiopulmonary exercise tests, cardiac magnetic resonance, liver biochemistry and imaging, detection of protein-losing enteropathy)., Conclusions: Our results showed that there is no unified surveillance approach for Fontan patients in Poland. The practice of care for adults differs from that of adolescents.

Atrial septal defect is the most common congenital heart lesion in adults. Although atrial septal defect closure is recommended in those with right heart enlargement or paradoxical embolism, data informing such indications in adults are quite limited. This population has many unique characteristics and needs. In recent years, significant progress has been made with regard to diagnostic modalities that facilitate the diagnostic workup of these patients. However, the decision‑making process, especially in selected adult patients population (advanced age, various concomitant diseases, poor LV function [both systolic and diastolic], pulmonary hypertension, concomitant arrhythmias or multiple defects, or deficient rims) is still not easy. Available data are predominantly focused on imaging endpoints and short‑term morbidity and mortality rather than long‑‑term. The evidence base for outcomes with or without defect closure comes from various studies with different observation periods. Moreover, the clinical experience in diagnosing and treating that subgroup of patients is inhomogeneous between individual physicians (cardiologists, imaging specialists, operators) and between small and large experience centers. In the view of the above, the joint group of experts from the Association of Cardiovascular Interventions and the Grown‑Up Congenital Heart Disease Section of the Polish Cardiac Society developed the following consensus opinion in order to standardize the principles of diagnosis, indications for treatment, methods of performing procedures, and tenets of postoperative care in Poland.

Background: Patients with complex congenital heart defects may have different hemodynamic prob-lems which require a variety of interventional procedures including angioplasty which involves using high-pressure balloons. After failure of conventional balloon angioplasty, cutting balloon angioplasty is the next treatment option available. The purpose of this study was to evaluate the safety and efficacy of cutting balloon angioplasty in children with different types of congenital heart defects., Methods: Cutting balloon angioplasty was performed in 28 children with different congenital heart defects. The indication for cutting balloon angioplasty was: pulmonary artery stenosis in 17 patients, creating or dilatation of interatrial communication in 10 patients, and stenosis of left subclavian artery in 1 patient., Results: In the pulmonary arteries group there was a significant decrease in systolic blood pressure (SBP) in the proximal part of the artery from the average 74.33 ± 20.4 mm Hg to 55 ± 16.7 mm Hg (p < 0.001). Distal to the stenosis there was an increase in SBP from 19.8 ± 3.82 mm Hg to 30.3 ± ± 13.3 mm Hg (p = 0.04). This result remained constant in the follow-up. In atrial septal defect/fenestra-tion group, cutting balloon angioplasty was performed after an unsuccessful classic Rashkind procedure. After cutting balloon angioplasty there was a significant widening of the interatrial communication., Conclusions: Cutting balloon angioplasty is a feasible and effective treatment option in different con-genital heart defects.

Muscular ventricular septal defects (VSD) located below the trabecula saepto-marginalis are difficult to approach for surgical closure through the tricuspid valve. We present the hybrid technique of perventricular closure of VSD in 2- and 5-year old children with complex congenital heart defects, employing an Amplatzer septal occluder, dedicated to muscular type of VSD. The procedures were performed during cardiopulmonary bypass in one patient and on beating heart in the other one. The perventricular device technique may be the method of choice for closing hard to reach muscular VSD.

We present 2 cases (4 and 17-year-old children) with aorta-vena azygos fistulae. In the fist child spontaneous closure was confirmed by clinical symptoms and aortography, in the second (with significant left-right shunt) successful closure with 10 x 8 mm Amplatzer Duct Occluder was obtained. These case reports show that the strategy of treatment of aorto-venous fistulae must be individualised.

Aim: To assess the early results of the pulmonary artery valve transcatheter implantation (PAVTI) in pts included into POL-PAVTI registry. Detailed medical and economic analyses were performed., Methods: Pulmonary artery valve implantation was performed in 14 pts (9 men), aged 16-31 (mean 24.6 +/- 4.8) years, with pulmonary homograft dysfunction after total repair of tetralogy of Fallot (4 pts), pulmonary atresia (2 pts), pulmonary stenosis (1 pt), common arterial trunk type I (1 pt), Ross procedure (3 pts) and TGA - Rastelli operation (3 pts). Eleven pts underwent in the past 2-5 surgical or/and catheter interventions. Indication for PAVTI was based on clinical evaluation and echocardiographic studies. Assessment of morphological and functional features of the right ventricle (RV) and homograft with the use of cardiac magnetic resonance (CMR) was performed in 10 cases. Pulmonary stenosis (max. pulmonary gradient 32-119, mean 72 +/- 28 mmHg) was observed in 13 pts and/or significant pulmonary regurgitation in 10 pts. The procedure was performed in general anesthesia. The deployment of a valved stent in the pulmonary valve position was preceded by a metal stent implantation. Results were evaluated by echocardiography two days after the procedure and one month later. Four patients were evaluated 6 months after procedure., Results: Time of the procedure varied 60-190 (mean 127 +/- 35) min, time of fluoroscopy ranged 12-31 (mean 21 +/- 11) min. PAVTI was successfully performed in all pts without serious complications. Patients were discharged from the hospital 48-293 (mean 120 +/- 71) h after procedure. Significant reduction of pulmonary gradient after the procedure assessed by echocardiography was observed on the second day (20-60, mean 38 +/- 12 mmHg, p < 0.0001) and one month (19-52, mean 34 +/- 9 mmHg, p < 0.0001). Mild pulmonary regurgitation was observed in 2 pts. In 5 pts evaluated 6 months after procedure haemodynamic parameters were unchanged; no late complications were observed. Average cost of the procedure including a price of the valve (82 000 PLN) was 98 000 PLN., Conclusions: Pulmonary artery valve transvascular implantation is an effective and safe method of non-surgical treatment for patients with homograft dysfunction. Cost-effectiveness is approvable.

Transcatheter implantation of pulmonary valve became a big step forward in the field of interventional cardiology. It is especially important in the patients with defects of the right ventricular outlet tract, because they were usually candidates for multiple surgical operations. We present first transcatheter replacement of pulmonary valve in 23-years-old man. The 'Melody' valve was implanted successfully. There were no complications and the patient was discharged in good condition.

Background: Exercise-induced hypertension following repair of the coarctation of the aorta (CoA) is a well known phenomenon. The most important functional parameters in the assessment of the effects of a surgical repair of CoA are the maximal pressure gradient in the descending aorta (GRAD) and systolic blood pressure (SBP). Results of treadmill exercise test using the Bruce protocol (treadmill test) and dobutamine stress echocardiography (DSE) were compared to determine utility of the DSE in the evaluation of the effects of surgical treatment of CoA in children., Methods: The study population comprised of 29 patients, including 20 males and 9 females (mean age 12 years) who underwent a surgical repair of CoA. Changes of the cardiovascular parameters including SBP, GRAD and heart rate (HR) during the treadmill test and DSE were compared., Results: During the treadmill test, SBP at peak exercise ranged from 120 to 230 (mean 163.7) mm Hg, GRAD ranged from 29 to 109 (mean 59.8) mm Hg, and HR ranged from 140 to 188 (mean 169) bpm. At the end of DSE, SBP ranged from 123 to 215 (mean 164.7) mm Hg, GRAD ranged from 29 to 113 (mean 55.4) mm Hg, and HR ranged from 76 to 155 (mean 111) bpm. We found positive correlations of SBP (r = 0.68, p < 0.001) and GRAD (r = 0.82, p < 0.001) values during both tests but no significant correlation for HR (r = 0.42, p = NS)., Conclusions: Dobutamine stress echocardiography is useful in the evaluation of the effects of surgical repair of CoA in children.

Background: Perimembranous ventricular septal defect (VSD) is the most common congenital heart defect. Percutaneous transcatheter closure of VSD is one of the greatest challenges in interventional cardiology., Aim: Presentation of our experience in transcatheter closure of perimembranous VSD., Methods: Eighteen patients were treated. Nine patients (group I) had VSD closed with implant Amplatzer Perimembranous VSD Occluder (PMVSDO) whereas the other nine had VSD closed with Muscular VSD Occluder (MVSDO). In the second group the presence of at least 4 mm rim from aortic valve was mandatory to undergo the procedure. Average patients age was 17.1 (3.2-40) years, defect diameter--4.7 (4-8) mm and Qp/Qs ratio--1.84 (1.5-4.6). Perimembranous interventricular septum aneurysm was noted in 5 cases. Only patients who had hemodynamically important defect (Qp/Qs ratio >1.5) were selected for interventional VSD closure. Patients with subarterial VSDs, pulmonary hypertension or/and aortic regurgitation were excluded. Ventricular septal defect closure was performed with standard techniques., Results: Procedures were completed successfully in 16 of 18 patients. There was no early or late implant embolisation. After the procedure in every case complete closure or important reduction of the shunt was observed. In the group I there was a trend towards more frequent occurrence of rhythm disturbances (p=0,08), including two cases with severe arrhythmias occurring during VSD closure requiring abandoning of procedure. In other 2 cases (patients age 12 and 14 years) in the second week after PMVSDO placement complete atrio-ventricular block occurred. In one patient sinus rhythm was restored after steroid treatment whereas another patient required pacemaker implantation. In group II mild nonprogressive tricuspid regurgitation was noted in 3 patients., Conclusions: Percutaneous perimembranous VSD closure is an interesting alternative to surgical treatment. In selected cases closure of the defect with muscular VSD implant is effective and safe.

Background: Muscular ventricular septal defects (VSD) are an important and difficult surgical problem. In the last few years a new alternative has emerged - possibility of VSD closure using percutaneous approach., Aim: To present our experience in percutaneous closure of congenital muscular and one posttraumatic VSD., Methods: We treated 10 patients - 7 children (age 0.8-7 years) and 2 adults (43 and 46 years) with congenital VSD, and one 18-year-old patient with posttraumatic VSD (knife stab). All the patients had a large haemodynamic shunt (Qp:Qs 1.9) and in all cases percutaneous closure attempt with an Amplatzer Muscular VSD Occluder (MVSDO) implant was undertaken. Five of 6 children with multiple muscular VSDs had in infancy previous pulmonary artery banding and one patient had complex heart disease: transposition of great arteries (dTGA), pulmonary stenosis (PS) and perimembranous VSD. All procedures were performed using the standard technique., Results: Eleven procedures were performed in 10 patients (one child had 2 attempts). Seven procedures were successful. In all cases a considerable reduction in flow or complete closure was achived. In one case, despite multiple attempts, VSD caniulation was ineffective and the procedure was abandoned. The patient had oblique VSD - morphology confirmed was later by the operating surgeon. The reason for the other 3 failures was early embolisation to the left ventricle and aorta. This complication was noted in 2 adult patients - one with congenital and one with post-traumatic VSD. In both cases the interventricular septum was thick (10 and 11 mm) and implants were removed with a bioptome or vascular lasso. Another embolisation occurred in a child with TGA - in this case the cardiac surgeon removed the implant from the aortic arch during Rastelli operation., Conclusion: Our experience acquired during muscular VSD closure with MVSDO indicates that the method is useful in children with isolated defects. Adult patients and children with a complex form of congenital defects should have morphology of MVSDO carefully evaluated and width of the interventricular septum measured to avoid potential implant embolisation.

A case of a 26-year-old man without continuity of blood flow in the level of the isthmus of the aorta is described. During interventional catheterization, we reconstructed this continuity and implanted a vascular stent.

Background: The aim of this paper is to present our own experience related to frequency of symptomatic tachy- and bradyarrhythmias in patients after transcatheter closure of interatrial defects (ASD) and patent foramen ovale (PFO) using Amplatzer plugs., Methods: Transcatheter closure of interatrial communications with Amplatzer devices was carried out on 739 patients in our center. Only patients with new symptomatic arrhythmias (who required pharmacotherapy, cardioversion or pacemaker implantation) were included in to the study. All patients who had had arrhythmias prior to ASD closure, such as supraventricular tachycardias (SVT) or atrial flutter/fibrillation (AF), were excluded., Results: New tachy- and bradyarrhythmias after implantation of Amplatzer devices were observed in 11 patients (1.5%). There were 9 patients (mean age 36.7 years) with atrial tachyarrhythmias (AF in 8 and SVT in 1 patients), which occurred between the first day and 3 months after implantation. Seven patients were treated initially by pharmacotherapy; in 2 of them sinus rhythm returned just after cardioversion. In other 2 patients cardioversion was performed as an initial therapy. In none of these patients, but one recurrence of tachycardia was observed; however, 7 of them had pharmacotherapy prolonged up to 1 year. In 2 patients, aged 15 and 16, complete atrioventricular (AV) block was observed 4.3 and 1.5 years after Amplatzer implantation, respectively. In the first patient intermittent second-degree AV block (Mobitz II) was observed before ASD closure. In both patients, a DDDR pacemaker was implanted., Conclusions: Transcatheter closure of ASD using Amplatzer devices is associated with a risk of new atrial tachyarrhythmias (usually early after the procedure and in older patients). The risk of conduction disturbances such as complete heart block, which can occur in late followup, is low. Thence, close long-term follow-up of these patients is obligatory.

Introduction: Restoration of blood flow through a previously occluded ductus arteriosus may occur in some patients. Treatment strategy in patients with such residual shunts has not yet been uniformly established., Aim: To present single-centre experience and to attempt to establish a strategy of management of patients with residual ductus arteriosus shunts following percutaneous closure., Methods: Of 352 patients who underwent percutaneous closure of ductus arteriosus, in 13 subjects complete closure failed (coils and Rashkind occluders were used in 10 and 3 patients, respectively). In these patients other percutaneous interventions aiming at total closure of residual shunt were attempted., Results: In 12 patients coils were inserted (one patient received two coils). Introduction of implant in one patient failed, but total occlusion of the shunt was confirmed one day after the procedure. Trivial residual shunt was observed in one patient after one-year follow-up., Conclusions: Percutaneous treatment of residual shunts within the ductus arteriosus is an effective and safe procedure. In our opinion identifying and treating such leaks is important, as it prevents complications and long-term need for antibiotic prevention of infective endocarditis. In the case of a small residual shunt, insertion of a coil seems to be the optimal therapy due to the low cost of the device, favourable design and high effectiveness. For patients in whom anatomy of the ductus arteriosus has been significantly changed, particularly in previously treated subjects, techniques using vascular loops or insertion using a catheter wedge may be helpful.

A case of percutaneous treatment of neonatal critical aortic stenosis is presented. The baby had endocardial fibroelastosis and borderline dimensions of left heart structures. Umbilical access for the anterograde approach with the use of coronary angioplasty catheter was successfully performed.

Background: Invasive procedures involving the atria may promote the development of iatrogenic cardiac arrhythmias., Aim: To analyse the prevalence of cardiac arrhythmias following transcatheter or cardiosurgical closure of the secundum type atrial septal defect (ASD)., Methods: The study group consisted of 91 patients, aged 2-18 years with haemodynamically significant ASD who underwent surgical (n=44) or transcatheter (Amplatzer occluder) (n=47) closure of ASD. Standard ECG and Holter ECG recordings, obtained before and after the procedure, were analysed. The follow-up duration ranged from 2.5 to 5.5 years. Cardiac arrhythmias were divided into benign or significant (requiring pharmacological therapy), early or late, and transient or permanent., Results: Cardiac arrhythmias were detected in 16 (36%) patients who underwent surgery compared with 1 (2.1%) patient who underwent transcatheter ASD closure (p<0.05). In surgically treated patients, arrhythmias were benign in 9 patients, significant in 7 children, early in 15 subjects, late in one patient, transient in 13 children and permanent in 3 subjects. One patient, who underwent transcatheter ASD closure, developed paroxysmal supraventricular tachycardia one day after the procedure, successfully terminated with verapamil., Conclusions: Transcatheter closure of ASD is associated with a lower risk of procedure-related arrhythmias than surgical treatment. However, longer follow-up in patients treated with transcatheter procedure is needed in order to draw definite conclusions.

Background: A significant aortic stenosis in children causes hypertrophy and destructive changes of the left ventricle (LV). Definitive treatment of this condition consists of aortic valve replacement. Temporary decompression of LV may be achieved by surgical valvulotomy or percutaneous balloon valvuloplasty (PBV). The use of the latter method remains controversial., Aim: To assess early and long-term effects of PBV of aortic stenosis in children., Methods: The study group consisted of consecutive 47 patients (age 4 months - 19 years, mean 8.3 years) with congenital aortic stenosis who were selected for PBV performed in a single centre., Results: PBV was performed in 46 patients and caused significant reduction of the trans-valvular pressure gradient (from 81.4+/-21.08 mmHg at baseline to 30.5+/-17.87 mmHg after PBV, p<0.0001). There were no peri-procedural deaths. One local complication occurred - damage of the femoral artery, which was treated surgically. In this patient PBV was not performed. In four patients the procedure was regarded as ineffective. During the mean follow-up of 38.4 (2-108) months there were no deaths, and eight patients underwent various types of cardiac surgery, mainly due to the progression of aortic regurgitation or lack of effects of PBV., Conclusions: PBV is an effective palliative method for the treatment of congenital aortic stenosis. This procedure enables the delay of surgery till adulthood. Aortic regurgitation is the most common complication, and sometimes requires early surgical intervention.